Download Duane Retraction Syndrome (Type 1A) in amale child

Duane Retraction Syndrome (Type 1A) in amale child –
Case Report
Dr.C.XavierJayaseelan, Assistant Professor
Dr.V.Panimalar, Assistant Professor
Dr.K.C.Parvatham, Professor & Head,
Department of Ophthalmology,
Saveetha Medical College & Hospital,
Thandalam,
CHENNAI.
Abstract
Duane retraction syndrome is a congenital form of strabismus characterized by horizonatal eye
movement limitation, globe retraction and palpebral fissure narrowing in attempted adduction.
DRS is present in 1% of strabismus patients. It is most commonly unilateral, but can be bilateral.
It affects the left eye more frequently than right eye and females more than males. Refractive
error commonly associated is hypermetropia. Here we are presenting a case of 15 year old boy
with Type 1A Duane's retraction syndrome in Right eye with myopia.
Key words : Duane’s retraction syndrome, Abduction , Type 1A
Introduction
Retraction Syndrome, also known as Stilling-Turk-Duane Syndrome, was originally described by
Alexander Duane in 1905. The syndrome was first described by ophthalmologists
JakobStilling(1887) and SiegmundTürk (1896), and subsequently named after Alexander Duane
who discussed the disorder in more detail in 1905.1,2,3,4,5,6
DRS is a congenital, incomitant ocular motility disorder characterized by abnormal function of
the lateral rectus muscle in the affected eye, together with retraction of the globe and narrowing
of the palpebral fissure on attempted adduction.6,7,8 Generally, the lateral rectus does not abduct
the eye, but instead contracts at the same time as the medial rectus on adduction. It is this
simultaneous cocontraction of the medial and lateral rectus muscles on attempted adduction that
causes the retraction of the globe and narrowing of the palpebral fissure when the eye is
adducted.9
DRS is present in 1% of strabismus patients.10 It is most commonly unilateral, but can be
bilateral.11 For unknown reasons, it affects the left eye more frequently (approximately 60%
incidence)11-16, and approximately 60% of patients with DRS are female.8 Although it is usually
sporadic, there could be up to 10% of familial cases mostly with autosomal dominant
inheritance.Hypermetropia of greater than +1.50 diopter was more frequent in DRS(71%).
Myopia and Emmetropia appeared in relatively equal amounts (15% and 14% respectively).17
It is usually sporadic, although there could be up to 10% of familial cases mostly with autosomal
dominant inheritance. Several autosomal dominant syndromes with dysmorphic features are
associated with DRS.18
Case Report
A 15 years old school boy attended the outpatient department with complaints of defective
vision in both eyes for few years and restricted outward movement of right eye since childhood,
according to his father. No history of head ache, nausea or vomiting was present. No history of
fever or trauma. No other members in family were affected. No ophthalmic consultations so far.
He was born out of a non-consanguous marriage with full term normal delivery. Antenatal,
intranatal and postnatal history was insignificant.
On examination, his unaided visual acuity in both eye was 6/36 which improved to 6/6 with
-1.50DS. He had restriction of abduction in RE (Fig 1) with normal adduction. It was associated
with narrowing of palpebral fissure and with retraction of the eye ball on adduction (Fig 3&4).
No upshoot or down shoot of eye was noted. He had Esotropia of 5 prism diopter in RE (Fig 2),.
Very mild degree of head turn towards right eye was noted which was cosmetically insignificant.
Binocular single vision was maintained.Fundus examination in both eyes showed normal disc,
vessels and macula, periphery was normal.The associated ocular features of DRS (Table I) were
not seen. Systemic abnormalities known to be associated with DRS were also not present.
Fig 1
Fig 2
Fig 3
Fig 4
Discussion:
Duane's syndrome is a congenital and non-progressive strabismus syndrome. It results from
an absent or dysplastic abducens motor neurons with aberrant innervations of the lateral rectus
muscle by the oculomotor nerve, from failure of normal development of the pontineabducens
nucleus or nerve resulting in failure of the normal innervation of the lateral rectus muscle on the
affected side. At the same time, an aberrant branch of the oculomotor nerve innervates the lateral
rectus muscle. Thus, globe retraction results from co-contraction of the medial and lateral rectus
muscles on attempted adduction
Huber has classified Duane's syndrome in to three types17(Table 1). Type 1 DRS, Type 2 DRS
and Type 3 DRS. Among them Type 1 is most common with 70-80% prevalence.
Hubers classification of DRS (Table 1)





Type - 1 (70%-80%)








Type - 2 (about 7%)






Type - 3 (about 15%)


Inability to abduct
Normal or minimal defect in adduction
Esotropia with head straight
A or V pattern
Usually updrift or a downdrift of
affected eye on adduction or
attemptedabduction
Globe retraction and palpebral-fissure
narrowing on adduction
Usual face turn to affected side
Electromyography shows absence of
electrical activity in the lateral rectus
muscle on abduction but paradoxical
electrical activity on adduction
Inability to abduct
Normal or minimal defect in adduction
Esotropia with head straight
A or V pattern
Usually updrift or a downdrift of
affected eye on adduction or attempted
abduction
Globe retraction and palpebral-fissure
narrowing on adduction
Usual face turn to affected side
Electromyography shows absence of
electrical activity in the lateral rectus
muscle on abduction but paradoxical
electrical activity on adduction
Inability ability to abduct and adduct
Globe retraction and palpebral-fissure
narrowing on attempted adduction
Possible upshoot and downshoot on
adduction
Straight or nearly straight head position
The electromyogram demonstrates
cocontraction of the horizontal rectus
muscles on both adduction and
abduction
A modification of Huber’s classification was proposed byAluwalis , Gupta, Goel and Khurana
based on the deviation in primary position of gaze17. This is relevant for only DRS Type 1
because DRS Type 2 is always Exotropia in primary position and DRS Type 3 is always
Orthotropia in primary posiyion. The proposed classification is as follows: DRS Type 1A
(esotropia in pp), DRS Type 1B (exotropia in pp) and DRS Type 1C (orthotropia in pp).So based
on the above classification we diagnosed the patient to have DRS Type 1A in right eye.
As already mentioned it is more common in females and left eye is more commonly involved
with hypermetropia being the most common refractive error associated , we are presenting a case
of a male child with right eye Duane retraction syndrome type 1A with myopia as the refractive
error.
Treatment options include correction of the refractive error, treatment of amblyopia and surgical
correction. Surgical correction is required for patients with significant head turn, strabismus in
primary gaze, and significant upshoot and downshoot on adduction.19,20 Surgical treatment has its
limitations as it does not assure complete clinical recovery.
As the patient had very mild head turn with no amblyopia with preserved binocular single vision
we opted for conservative line of management and prescribed him glasses. Regular follow up
was advised and no worsening of symptoms noticed till date.
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